Successful treatment of scleromyxedema with dexamethasone cyclophosphamide pulse therapy

Cyclophosphamide is a chemotherapeutic agent which was first discovered in experimental tumours in rats, and it has since been widely used to treat malignancies and severe manifestations of various auto-immune diseases. High-dose chemotherapy and continuous daily oral regimens are associated with significant toxicity profiles, but i.v. pulsed regimens have lowered the rates of adverse effects in rheumatological studies. Cyclophosphamide has been shown to be useful in the treatment of severe autoimmune conditions due to its powerful immunosuppressive ability; however, it remains a relatively underused modality in dermatology. This article reviews the current literature on cyclophosphamide and its clinical applications in dermatology.

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“…[114][115][116] One case report describes clinical improvement by the third cycle of DCP, and near total softening of the skin by 24 cycles. 117 …”

Section: Chp or Sc Panniculitic T-cell Lymphoma (Sptcl)mentioning

confidence: 97%

Cyclophosphamide in dermatology

Kim

Chan

2016

Aust J Dermatology

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“…Causal treatment of scleromyxedema is unavailable, as the aetiology is still unclear [ 9 ]. The severe course of the disease requires very aggressive treatment and long-term maintenance therapy is necessary in most cases [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Scleromyxedema: a rare disorder and its treatment difficulties

Koronowska¹,

Osmola‐Mańkowska²,

Jakubowicz³

et al. 2013

pdia

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Scleromyxedema is a rare progressive cutaneous mucinosis, usually associated with a systemic involvement and paraproteinemia. Its aetiology remains unknown. The therapeutic options include numerous treatment modalities, however, no standard treatment exists as the rarity of this disease prevents the execution of controlled therapeutic trials. This paper reports a case of a 38-year-old male with progressive scleromyxedema associated with gammopathy. Initially, the patient was treated with prednisolone and later etretinate was added to the therapeutic schedule with quite good clinical improvement. However, after 6 months of treatment, several adverse effects were observed: hypercholesterolemia, hypertriglyceridaemia and cataract of the right eye. The patient was consulted by dermatologists in Warsaw and Gdansk as well as by a haematologist. The patient was excluded from oncological treatment. Melphalan therapy was not recommended as it is associated with very toxic side effects. IVIG treatment (intravenous immunoglobulin) was not initiated because of financial issues. As the disease progressed, treatment with plasmapheresis was introduced. The patient received 4 cycles of the therapy. It was well-tolerated by the patient and gave satisfactory, but temporary results. In order to obtain long-lasting improvement the patient was treated with IVIG (21.0 g/dose for 5 consecutive days). This treatment modality seems to have resulted in a more stable improvement.

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“…Historically, melphalan therapy has been the treatment of choice for this condition with multiple reports of benefit, but significant toxicity appears frequent (61)(62)(63)(64). Case reports cite variable improvement with other immunosuppressants including cyclophosphamide and cyclosporine (65)(66)(67)(68). There are multiple cases describing some benefit using thalidomide, although there remains a legitimate concern for the development of disabling peripheral neuropathy (68)(69)(70)(71)(72).…”

Section: Treatment and Prognosismentioning

confidence: 99%

Scleroderma-like Fibrosing Disorders

Boin¹,

Hummers²

2008

Rheumatic Disease Clinics of North America

109

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SynopsisMany conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology and disease associations may allow to distinguish these conditions from scleroderma and from each other. A prompt diagnosis is important to spare the patients from ineffective treatments and inadequate management. In this review, we highlighted nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy), eosinophilic fasciitis (Shulman's syndrome), scleromyxedema and scleredema. These are often detected in the primary care setting and referred to rheumatologists for further evaluation. Rheumatologists must be able to promptly recognize them to provide valuable prognostic information and appropriate treatment options for affected patients.

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Successful treatment of scleromyxedema with dexamethasone cyclophosphamide pulse therapy

Cited by 18 publications

References 0 publications

Cyclophosphamide in dermatology

Cyclophosphamide in dermatology

Scleromyxedema: a rare disorder and its treatment difficulties

Scleroderma-like Fibrosing Disorders

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